A Pediatric Case of Primary Sjogren's Syndrome Associated with Nephrogenic Diabetes Insipidus and Renal Tubular Acidosis

Choi, Jong Won; Jung, You Jin; Suh, Jin Soon; Park, So Hyun; Koh, Dae Kyun

doi:2012.16.2.126

J Korean Soc Pediatr Nephrol > Volume 16(2); 2012 > Article

J Korean Soc Pediatr Nephrol 2012;16(2): 126-131. doi: https://doi.org/10.3339/jkspn.2012.16.2.126

신성요붕증과 신세뇨관산증을 동반한 일차성 쇠그렌 증후군의 1례

최종원, 정유진, 서진순, 박소현, 고대균

¹가톨릭대학교 의과대학 소아과학교실
²가톨릭대학교 의과대학 소아과학교실
³가톨릭대학교 의과대학 소아과학교실
⁴가톨릭대학교 의과대학 소아과학교실
⁵가톨릭대학교 의과대학 소아과학교실

A Pediatric Case of Primary Sjogren's Syndrome Associated with Nephrogenic Diabetes Insipidus and Renal Tubular Acidosis

Jong Won Choi, You Jin Jung, Jin Soon Suh, So Hyun Park, Dae Kyun Koh

¹Department of Pediatrics, The Catholic University of Korea, College of Medicine
²Department of Pediatrics, The Catholic University of Korea, College of Medicine
³Department of Pediatrics, The Catholic University of Korea, College of Medicine
⁴Department of Pediatrics, The Catholic University of Korea, College of Medicine
⁵Department of Pediatrics, The Catholic University of Korea, College of Medicine

Received: March 18, 2012; Revised: June 15, 2012. Accepted: June 20, 2012.

ABSTRACT

Sjogren's syndrome (SS) is an autoimmune disorder primarily affecting the salivary and lacrimal glands. In addition, extra-glandular manifestations involving the lungs, liver, kidneys, pancreas, skin and central nervous system were reported in patients with SS. These extra-glandular manifestations are not rare in adult patient, but are very rare in pediatric SS. Renal manifestations are relatively common in adult SS, but are rarely reported in childhood SS. We experienced a girl with primary SS manifested with nephrogenic diabetes insipidus and renal tubular acidosis.

Key words: Sjogren's syndrome | Diabetes insipidus | Renal tubular acidosis | Child